ReviewFrontiers in cell and developmental biology2024
Desmoplastic small round cell tumor: from genomics to targets, potential paths to future therapeutics.
Review in Frontiers in cell and developmental biology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
13 citing papers in PubMed.
- Review
- Desmoplastic Small Round Blue Cell Tumor in a Young Adult Man: A Rare Case of Abdominal Sarcoma.The American journal of case reports · 2026Article
- CRISPR-edited cell lines for studying rare desmoplastic small round cell tumors.Annals of medicine and surgery (2012) · 2026Article
- Antitumor Activity of Trastuzumab Deruxtecan in Pediatric Solid Tumors with Variable HER2 Expression.Molecular cancer therapeutics · 2026Article
- Radiologically Occult Desmoplastic Small Round Cell Tumor in a 36-Year-Old Man: A Case Report.Cureus · 2026Article
- Management and survival outcomes of desmoplastic small round cell tumor: a retrospective cohort study from a tertiary cancer center.BMC cancer · 2025Article
- WT1 gene: a potential therapeutic target for multiple cancer treatment strategies.Clinical and experimental medicine · 2025Review
- Prevalence of locoregional and distant lymph node metastases in children and adolescents/young adults with soft tissue sarcomas: a Bayesian meta-analysis of proportions.EClinicalMedicine · 2025Article
- Ewing Sarcoma, Desmoplastic Small Round Cell Tumor, and Other Round Cell Sarcomas.Hematology/oncology clinics of North America · 2025Review
- Advancing Precision Medicine: The Role of Genetic Testing and Sequencing Technologies in Identifying Biological Markers for Rare Cancers.Cancer medicine · 2025Review
- High expression level of ERBB2 and efficacy of trastuzumab deruxtecan in desmoplastic small round cell tumour: a monocentric case series report.ESMO open · 2025Article
- Review
- Unresectable Desmoplastic Small Round Cell Tumor: A Case Report and Review of the Literature.Case reports in oncologyArticle
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
6 authors.
Funding
Abstract
Desmoplastic Small Round Cell Tumor (DSRCT) is a highly aggressive pediatric cancer caused by a reciprocal translocation between chromosomes 11 and 22, leading to the formation of the EWSR1::WT1 oncoprotein. DSRCT presents most commonly in the abdominal and pelvic peritoneum and remains refractory to current treatment regimens which include chemotherapy, radiotherapy, and surgery. As a rare cancer, sample and model availability have been a limiting factor to DSRCT research. However, the establishment of rare tumor banks and novel cell lines have recently propelled critical advances in the understanding of DSRCT biology and the identification of potentially promising targeted therapeutics. Here we review model and dataset availability, current understanding of the EWSR1::WT1 oncogenic mechanism, and promising preclinical therapeutics, some of which are now advancing to clinical trials. We discuss efforts to inhibit critical dependencies including NTRK3, EGFR, and CDK4/6 as well as novel immunotherapy strategies targeting surface markers highly expressed in DSRCT such as B7-H3 or neopeptides either derived from or driven by the fusion oncoprotein. Finally, we discuss the prospect of combination therapies and strategies for prioritizing clinical translation.
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What Socratic holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.